Anaesthetic Consideration in Macroglossia Due to Lymphangioma of Tongue: A Case Report

Summary Successful airway management of an infant or child with macroglossia prerequisites recognition of a potential airway problem. We describe our experience with a debilitated 13-year-old girl who presented with severe macroglossia, secondary to lymphangioma of the tongue. Along with the social discomfort she had inability to speak, eat or drink properly and exposure-induced dryness. Such patients are a challenge for the anaesthesiologists due to the anticipated difficult intubation associated with the oral mucosa occupying lesion. It also becomes pertinent to rule out any of the associated congenital anomalies. The importance of a thorough preoperative evaluation and attention to difficult intubation and maintenance of airway is emphasized. We endeavor to review the available literature regarding patient's perioperative management of such patients.


Introduction
A child with markedly enlarged tongue presents a unique challengeto the anaesthesiologists. Techniques for managing difficult airway in children are different from those used in adults. The anaesthesiologist and otorhinolaryngologists team should plan and execute the perioperative airway management. We present a case of recurrent lymphangioma of tongue leading to macroglossia in a 13-year-old girl who had presented for hemiglossectomy.
The first accurate description of lymphangioma was given by Virchow in 1854. Lymphangiomas are benign hamartomatous tumors of the lymphatic channels.They presentas developmentalmalformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels 1 . They can also occurin association with hemangioma.
Lymphangiomas have a marked predilection for the head and neck region, which accounts for about 75% of all cases and about 50% of these lesions are noted at birth and around 90% develop by 2 years of age 2 . They are known to be associated with Turner's syndrome, Noonan's syndrome, trisomies, cardiac anomalies, fetal hydrops, fetal alcoholsyndrome, and Familialpterygium colli 2 .
Oral lymphangiomas may occur at various sites butthey form most frequentlyon theanterior two-thirds of thetongue, whichoften resultin macroglossia.It can also present in the palate, buccal mucosa, gingiva, and lip 3,4 .

Case report
A 13-year-old female child presented to otorhinolayrngology department with enlargement of tongue for the last 1 year. It was insidious in onset. She gavea history of progressive difficulty in ingesting semi solid food. The girl had been operated for the same pathology, atthe age of four months under generalanaesthesia after oro-tracheal intubation. There were no records of any perioperative complications then.
A thorough preoperative examination was done prior to surgery. The girlwas poorly nourished, under weight (18 kg), anxious, embarrassed, apprehensive, and was unable to speak comprehensively. There was no history of respiratory difficulty, trauma, pain, bleeding or sudden increase in the size of lingual swelling. She could take only liquid diet. A good rapport was developed with her and she was explained in vernacular language the need for surgery and what she should expect in the operatingroom. Extensiveexamination of the other body systems revealed no relevant medical problems.
Local examination revealed a diffusely enlarged tongue;protruding andkeepingthemouth permanently open (Fig 1& 2).Ulcerations overthe dorsum of tongue on anterior part were present. The oro-dental hygiene was poor with the lower teeth completely compressed inside the swollen gums. Mouth opening, inter-incisor gap and Mallampatti grading could not be elicited due to the enlarged tongue. On palpation the tongue was tender and firm in consistency.All other congenital abnormalities were looked for and ruled out by the pediatrician. Preoperative blood analysis revealed a haemoglobin of 10mg/dL and a haematocrit of 30%. The serum electrolytes, ECG, and chest X-ray were normal. Fine needleaspiration cytologyof thelingualswelling yielded only blood, hence a clinical diagnosis of hemangioma was made. The child was to be taken up for V-glossoplasty.
Premedication wasgiven inthe form of midazolam 1mg, glycopyrrolate 0.2mgand fentanyl 30 g intravenously prior to shifting her to operation room. All the preparation for anticipated difficult intubation/ventilation andtracheostomy were kept ready.Monitoring was initiated with continuous ECG, arterialoxygen saturation, temperature and non invasive blood pressure monitoring. Thesurgeons wereasked toremain standby, in case if tracheostomy was required. Both the nostrils were instilled with xylometazoline d ro ps. Preoxygenation was initiated using a large anatomical facemask(number 4). The focus was on "Awake intubation", we did not give any thing for induction... as that wouldhave compromisedthe airway. The fibreoptic scope PentaxF1-10P2 wasintroduced through the right nostril (Fig 3). In the oralcavity it was difficult to ma- nipulatethe fibrescope due tothe enlargedtongue. The neck of the patient had to be flexed for proper visualization of the glottis and then the cuffed endotracheal tube (ETT) number 6.5 was guided under vision into the trachea. Neuromuscular blockade was achieved with rocuronium and the ETTwas secured after confirming bilateral air entry (Fig4). She was maintained on propofol (10mg.kg -1 .hr -1 ) and fentanyl infusion (10 g.hour -1 ) alongwith oxygenand nitrous oxide (ratio of 33:67).
Occasionallesions demonstrate proliferation of lymphaticchannels withanother connectivetissue component, prim arily sm ooth m uscle cells (lymphangiomyoma). Itis derivedembryologically from five primitivebuds developingfrom the venous system which include paired jugular sacs, paired posterior sacs and a single retroperitoneal sac 5 . Cervical lesions in a child can cause dysphagiaand airwayobstruction which is rare in adults 6 . In the present case, the swelling was noticed since birthfor which she had undergone palliative surgery at the age of four months. There was resurgence of the lingualswelling in the last one year.
The anterior two-thirds on the dorsal surface of tongue is themost commonsite for intra-orallymphangiomas leadingto macroglossia 3,4 . These patients tend to have speech disturbances, poor oral hygiene, and bleeding from tongue associated with oral trauma 7 . In our case, macroglossia resulted in lesions on the dorsal surface of tongue, improper phonation and poor oral hygiene.
The various treatment modalities for lymphangioma are surgical excision, radiation therapy, cryotherapy, electrocautery, sclerotherapy, steroid administration, embolization and ligation 6,8 ,laser surgery with

Fig 4 Endotracheal tube secured after proper confirmation of its placement
The inverted V shaped anterior half of the tongue was removed. The tumor had large cystic spaces filled with lymphlikefluid.The partof tumorgoingintothe base of the tonguewas injectedwith sclerosingagent i.e.25% dextrose.The tonguewas thensutured inmidlinetogive it normalshape.The surgerylasted for three hoursand the totalblood loss was150ml.Atthe endofsurgerythechild was allowed to return back to spontaneous respiration and neuromuscular blockadewasreversedafter adequate return of muscle power and respiratory tidalvolume. It wasdecided to keep herelectively intubatedand thereafter put on T-piece connected to the endotracheal tube with oxygen support 6L/minute. Shewas shiftedto the post anaesthesia care unit where she was kept sedated withpropofolandfentanyl. Thetrachea wasextubated on the second postoperative day. She maintained her vitals and arterialoxygen saturationwithin normallevels without any support.
Nd-YAG 9-11 , CO 2 12,13 ,and radio-frequency tissue ablation technique 14 . Surgical excision is the preferred treatment for cystic hygroma but complete removal is not possible because of the multiple fingerlike projections in the surrounding tissues 15,16 .
Since lymphangiomais primarilya diseaseof childhood, the paediatricdentist mightbe the first healthcare professional to encounter this lesion. An early diagnosis and intervention would help in reducing functional and psychological disturbances and alsocosmetic disfigurement.A complete and frank discussion with the parents (and child ifappropriate) should always include the anaesthetic andsurgical protocolalongwiththe associated risks. It is pertinent that the possibility of tracheostomy and, indeed, of failure to secure the airway should bementioned.
Awake intubation may not be easilyperformed in children since cooperation is quintessential. Premedication and preoxygenation should be followed by inhalation of either halothane or sevoflurane,in a spontaneously breathingpatient. Muscle relaxants should be withheld untilthe airway is secured. Intubation should be performedunder deep inhalationalanaesthesia. Use of amuscle relaxantduringinduction ofanaesthesia may result in a situation wherewe may land up in either a difficult to ventilate and difficult to intubate scenario, and may therefore warrant securing of a surgical airwayrapidly.Hence maintenanceof spontaneousbreathing allows a way out, should there be a problem in securing the airway 17,18 .
Visualization of thelarynx isbetter in deeper planes of anaesthesia. If difficult for whatever reasons, the anaesthesiologistmust have a secondary plan of how to proceed. If the surgicalrequirement is not pressing, onemust considerpostponingthe procedure, but if the procedure is essentialthen alternative means must be available to accomplish endotrachealintubation.Inthese groups of patients, certain maneuvers using conventional equipment do not always succeed, hence fibreoptic intubation techniques areoftennecessary 18 .
Adult fibreoptic bronchoscopes have an outerdi-ameter of around 3.5-4.0mm and thus can take realistically a size 4.0-4.5 endotracheal tube loaded onto them. Ultra thin fibrescopes have an outer diameter of 2.2 mm so a 2.5 mm endotracheal tube can be railroaded over them 18 . The opticalquality of these scopes is good butit hasno suctionchannel and secretionshave to be aspirated with a suction catheter 18 .
Anaesthesia can be maintained via a nasalairway or viaa specially adapted facemask.The bronchoscope canthen beinserted into the mouthand thelarynx visualized.The laryngeal mask airway can also be used. Use of this devicein anaesthetic practice can avoid the need for intubation 19 ,butshould intubation be deemed necessary it provides a superbairway conduit. The laryngealmask airwayin paediatricpatients with difficult airway is an excellent aid to visualize the larynx and endotracheal intubation 20 .
Blind techniques are possible with either a gum elastic bougie or an endotracheal tube 21 . Fibreoptic techniques depend on adaptation either of the laryngealmaskairway (split 22 or shortened 23 ) or of the mode of endotrachealpassage (telescopingthe tube over the fibrescope 24 or a wire technique 25 ). Shortening the laryngealmaskairway and splitting areother methods to the same end, allows the anaesthesiologist to advance the endotrachealtube through the laryngeal mask airway which can then beremoved withouthazardingthe tube.
The guidewire technique allows th e anaesthesiologisttoinsert a conventionaladult fibreoptic bronchoscope to gain a view of the larynx and use the suction facility. The suction channelcan then be employed to facilitate passageof a long guidewire into the trachea. This avoids the need to preload an endotracheal tube onto the fibrescope and railroad the tube into the trachea through the laryngeal mask airway, a difficult procedure, and even then the laryngealmask airway must be removed to allow properfixation of the tube.
When the patient is breathing deeply, spontaneously, on sevofluraneor halothane, the fibreoptic bron-choscope is introduced and a view of the cords is obtained. Lidocaine 2-3 mg.kg -1 may be sprayed via the suction channelof thefibreoptic scope onto the cords. The fibreoptic scope is manipulatedthrough the cords into the trachea until the bifurcation ofthe trachea is visible. Fibreoptic intubation should not be undertaken lightly in children.